2026-04-04T12:24:48Z https://www.peertechzpublications.org/oai-pmh

oai:www.peertechzpublications.org:10.17352/ahcrr.000035 2022-01-20 PTZ.AHCRR:VOL7

Combined treatment with vemurafenib and cobimetinib in Langerhans cell histiocytosis and Erdheim-Chester disease overlap syndrome: A case Report Hidalgo-Soto Marta Poza-Santaella María Pita-Suárez Daniel Calbacho-Robles María Pina-Sánchez José González-Medina JoséBaumann Tycho Stephan<p>Histiocytoses are clonal disorders diseases derived from the monocyte-macrophage lineage. The Erdheim–Chester Disease (ECD) and Langerhans Cell Histiocytosis (LCH) may occur in association with overlapping clinical, histopathological and molecular features, harboring somatic MAP2K1 mutations in more than 50% of patients. BRAF and MEK inhibitors have shown to be efficacious in ECD and LCH, including responses in patients with CNS involvement.</p><p>This case report describes a 59-year-old woman who presented with vemurafenib-refractory ECD/LCH overlap syndrome treated with vemurafenib/cobimetinib dual therapy, with rapidly progressing neurological involvement after its initiation.</p><p>Although targeted therapy plays a crucial role in the treatment of histiocytosis, only anecdotal clinical cases treated with dual therapy have been reported in ECD or LCH and collaborative trials are needed to improve outcomes.</p> Archives of Hematology Case Reports and Reviews - Peertechz Publications 2022-01-20 Case Report https://doi.org/10.17352/ahcrr.000035 en Copyright © Hidalgo-Soto Marta et al.